IJAR.2020.152

Type of Article:  Case Report

Volume 8; Issue 3.2 (August 2020)

Page No.: 7644-7648

DOI: https://dx.doi.org/10.16965/ijar.2020.152

A RARE CASE OF HYDRANENCEPHALY: A CASE REPORT

Zafar sultana *1, Anantha kumari 2, Habib G Pathan 3.

*1 Associate Professor of Anatomy, Deccan College Of Medical Sciences, Hyderabad, Telangana, India.

2 Professor and HOD of Anatomy, DCMS, Hyderabad, Telangana, India.

3 Professor of Pediatrics and Pediatric Neurologist, DCMS, Hyderabad, Telangana, India.

Corresponding author: Dr Zafar Sultana, Associate professor of Anatomy, DCMS, Hyderabad, , Telangana, India.  E-Mail: drszafarm@gmail.com

ABSTRACT

Hydranencephaly  is a rare congenital abnormality characterized by  replacement of the cerebral hemispheres by a large cerebrospinal fluid pool. It is thought to be caused by occlusion of bilateral internal carotid arteries in the fetal life mainly during the second trimester due to a variety of causes. It is one of the recognized forms of brain malformations which is usually associated with intrauterine fetal demise and is therefore rarely seen in postnatal life. It is a rare entity with a reported incidence of less than 1 per 10,000 live births. Hydranencephaly is an isolated abnormality with a severe prognosis, affecting the cerebral mantle. Midbrain is usually not involved. Differential diagnosis is mainly relevant when considering severe hydrocephalus, poroencephalic cyst and alobar holoprosencephaly. Clinical features include intact brainstem reflexes without evidence of higher cortical activity. Infants with this condition are normal at birth however, after a few weeks, they usually become irritable and have increased muscle tone and, after a few months of life, seizures and hydrocephalus (excessive accumulation of CSF in the brain) may develop. Other symp­toms are growth retardation, impaired vision, deafness and spastic paralysis. Due to the late onset of most symptoms and signs, the diagnosis may be delayed.

Key words: Hydranencephaly, Internal carotid artery occlusion, cerebrospinal fluid, Hydrocephalus, porencephalic cyst.

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Cite this article: Zafar sultana, Anantha kumari, Habib G Pathan. A RARE CASE OF HYDRANENCEPHALY: A CASE REPORT. Int J Anat Res 2020;8(3.2):7644-7648. DOI: 10.16965/ijar.2020.152